A case of West syndrome well controlled by very short and low-dose ACTH therapy

The case of a 5-month-old boy with tuberous sclerosis and West syndrome is reported. Tonic spasms were noted from the age of 4 months. High-dose pyrid oxal phosphate could not control the seizures completely. Very short and lo w-dose adrenocorticotropic hormone (ACTH) therapy (i.e. 0.011 mg/kg per dos e, 12 times in 20 days) controlled the seizures, while pyridoxal phosphate was on. Early tapering of ACTH was successfully done while abnormal electro encephalogram (EEG) findings remained. Although side effects such as hypert ension and brain shrinkage were transiently observed, both the cognitive an d seizure prognoses were excellent at the age of 3 years and 2 months. The good response to a small dosage of ACTH might be due to some responsiveness of the high-dose pyridoxal phosphate and the underlying cause of tuberous sclerosis with normal development before onset. The present case illustrate s that the duration and dosage of ACTH therapy in West syndrome should be m odified according to the individual's requirements.